POS0164 CHARACTERIZATION OF PULMONARY MANIFESTATIONS OF SJÖGREN SYNDROME: A MULTICENTER RETROSPECTIVE STUDY
Authors: L. Meudec, C. Marques, P. A. Juge, R. Dhote, A. L. Fauchais, E. Dernis, O. Vittecoq, A. Saraux, J. E. Gottenberg, E. Hachulla, V. Le Guern, P. Dieudé, M. P. Debray, A. Beurnier, X. Mariette, R. Seror, G. Nocturne
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Primary Sjögren syndrome (pSS) is a systemic autoimmune disease that may complicate with pulmonary manifestations in up to 16% of patients [1], including interstitial lung disease (SS-ILD) and airway disease (SS-AD). <h3>Objectives</h3> To assess the associated factors with SS-ILD and SS-AD. Secondary objectives: to describe these manifestations and to investigate their prognosis. <h3>Methods</h3> We performed a retrospective multicentric study involving 9 French centers. We included pSS patients fulfilling the ACR/EULAR 2016 criteria and having a pulmonary disease associated with pSS evidenced by at least one clinician and one computed tomography (CT) report. We collected clinical and biological data at the visit giving access to the most exhaustive collection, pulmonary function test (PFT) and CT scans of the patients. CT scans were reviewed by a radiologist specialist in thoracic diseases. SS-ILD were considered progressive if there were at least a 10% decrease of the forced vital capacity (FVC) between 2 consecutive measurements. SS-ILD and SS-AD were compared to pSS controls with no history of pulmonary involvement, matched on age and disease duration with a 2/1 ratio. <h3>Results</h3> We included 56 SS-ILD, 31 SS-AD and 174 pSS controls. Comparison of SS-ILD, SS-AD and pSS controls is shown in <b>Table 1</b>. We found that SS-ILD and SS-AD had higher disease activity (ESSDAI) and B cell biological markers at visit time. Incident lymphomas were more prevalent in SS-ILD. SS-ILD were mostly nonspecific interstitial pneumonia (NSIP, n=16, 36%), usual or probable usual interstitial pneumonia (UIP, n=8, 18%), indeterminate for UIP (n=8, 18%) and lymphoid interstitial pneumonia (LIP, n=4, 9%). Fibrosis features were observed in 43% of cases. 44% of SS-ILD were progressive, independently of pSS characteristics and CT pattern, despite any significant evolution of PFT parameters of overall SS-ILD within 7 years follow-up (<b>Figure 1A</b>). We indirectly assessed the impact of the first line of systemic treatments in SS-ILD comparing PFT evolution of treated (43%) and untreated patients. We found that treated SS-ILD tended to have reduce PFT deterioration than non-treated patients with a 6 years follow-up [especially FVC and total lung capacity (TLC)], with a trend to improved FVC and forced expiratory volume (FEV1) with cyclophosphamide and rituximab (<b>Figure 1C-D</b>). SS-AD were mostly diffuse, associating bronchiolitis and bronchiectasis in 60% of cases. Here again, we did not find a significant worsening of PFT parameters with 9 years follow-up in SS-AD (<b>Figure 1B</b>), despite CT abnormalities worsening in 41% of cases at 6 years. <h3>Conclusion</h3> SS-ILD are usually fibrosing and progressive manifestations of pSS, associated with disease activity, incident lymphoma and B cell biological markers. Despite the absence of randomized trials in SS-ILD, our results suggest an effect of the therapeutics. SS-AD are also associated with the disease activity and seems to progress slowly. <h3>Reference</h3> [1]Ramos-Casals et al., Rheumatology. 2015;54(12):2230‑8. <h3>Acknowledgements</h3> The authors would like to thank all the investigators of the ASSESS cohort for their contribution to this study. <h3>Disclosure of Interests</h3> None Declared.